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Oncology Boards > Malignant Heme > Flashcards

Malignant Heme Flashcards

1
Q

Subsequent Line Treatment of CML with acquired T315l mutation

A

Ponatinib + Asciminib
- Panatinib more arterial occlusive events
(less commonly used: Omacetaxine)

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2
Q

Used in patients with myelofibrosis and severe thrombocytopenia

A

Pacritinib

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3
Q

Induction regimen of choice for patients with treatment related AML and chromosome 5,7 deletions

A

Liopsomal doxorubicin + cytarabine

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4
Q

Treatment of newly diagnosed AML:
a. without drive mutation
b. with FLT3 mutation
c. with IDH1/2 mutation

A
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5
Q

CMV prophylaxis after transplant:
- 1st Line
- 2nd Line

A

1st Line- Letermovir
2nd Line- Maribavir

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6
Q

GELF Criteria for FL

A

GELF Criteria for High Tumor Burden in Follicular Lymphoma
1. Nodal or extranodal masses ≥ 7 cm in diameter
- Any single lymph node or extranodal mass that is 7 cm or larger.
2. Three or more nodal sites, each ≥ 3 cm
- Presence of three or more lymph nodes, each at least 3 cm in size.
3. B symptoms
4. Splenomegaly
5. Pleural effusions or ascites
6. Leukemia phase: Circulating lymphoma cells (leukemic involvement) with an ALC ≥ 5 x 10⁹/L.
7. Cytopenias: Hemoglobin level < 10 g/dL, or platelet count < 100 x 10⁹/L due to bone marrow involvement.

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7
Q

Treatment of relapsed/refractory DLBCL with ECOG 0-1

A

IF MORE THAN 12 MONTHS SINCE RELAPSE: CHEMO SENSITIVE
1. Salvage chemotherapy (2-3 cycles) followed by restating PET and if response, proceed with high dose chemotherapy and ASCT.

Common salvage chemotherapy regimens include:
- R-DHAP (rituximab, dexamethasone, cytarabine, cisplatin)
- R-ICE (rituximab, ifosfamide, carboplatin, etoposide)
- R-GDP (rituximab, gemcitabine, dexamethasone, cisplatin)

If they achieve a sufficient response (usually complete or partial remission), they proceed to high-dose chemotherapy followed by ASCT.

IF MORE THAN 12 MONTHS: PRIMARY REFRACTORY/EARLY RELAPSE
- CAR-T cell therapy preferred with axicabtagene ciloleucel or lisocabtagene maraleucel

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8
Q

Common Positive CD Markers for B cell disorders
a. CLL
b. Mantle Cell
c. Marginal Zone
d. Follicular

A

a. CLL: (+) CD5, CD19, CD23
b. Mantle Cell: (+) CD5, CD19, CD20
c. Marginal Zone: (+) CD11c, CD19, CD20
d. Follicular: (+) CD10, CD19, CD20

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9
Q

Treatment of CLL without TP53 mutation/17p deletion (category 1)
a. First Line (4)
b. Second Line (2)
c. Relapse after first line therapy if period of clinical remission

A

a. First Line:
- Acalabrutinib +/- obinutuzumab
- Venetoclax +/- obinutuzumab
- Ibruitinib
- Zanibrutinib

b. Second Line:
- BTKi (Acala/Zanu-bruitinib)
- BCL2 inhibitor: venetoclax + rituxumab

C. Re-treatment with Venetoclax +/- obinutuzumab

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10
Q

Treatment of CLL WITH TP53 mutation/17p deletion
a. First Line (3)
b. Second Line (3)

A

First Line:
* Acalabrutinib ± obinutuzumab
* Venetoclax + obinutuzumab
* Zanubrutinib

Second Line:
* Acalabrutinib
* Venetoclax + rituximab
* Zanubrutinib

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11
Q

4th Line Therapy options for multiple myeloma (6)

A

–Selinexor + Dexamethasone [for patients refractory to 2 PIs, at least two IMiDs and anti-CD38 antibody]
–Idecabtagene vicleucel [Preferred]
–Ciltacabtagene autoleucel [Preferred]
–Teclistamab-cqyv [Preferred]
–Talquetamab-tgvs [Preferred]
–Elranatamab-bcmm [Preferred]

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12
Q

Treatment of multiple myeloma with Anti-CD38 Refractory disease (2)

A

– Carfilzomib/lenalidomide/dexamethasone (category 1)
– Pomalidomide/bortezomib/dexamethasone (category 1)

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13
Q

Treatment of multiple myeloma with Bortezomib-Refractory disease (4)

A

–Carfilzomib/lenalidomide/dexamethasone (category 1)
–Daratumumab/carfilzomib/dexamethasone (category 1)
– Daratumumab/lenalidomide/dexamethasone (category 1)
– Isatuximab-irfc/carfilzomib/dexamethasone (category 1)

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14
Q

Treatment of multiple myeloma with Lenalidomide-Refractory disease (4)

A

– Daratumumab/bortezomib/dexamethasone (category 1)
– Daratumumab/carfilzomib/dexamethasone (category 1)
– Isatuximab-irfc/carfilzomib/dexamethasone (category 1)
– Pomalidomide/bortezomib/dexamethasone (category 1)

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15
Q

Gene rearrangement associated with follicular lymphoma

A

Follicular lymphoma is associated with t(14;18). This translocation juxtaposes BCL2 found on chromosome 18 to IgH found on chromosome 14. BCL2 has anti-apoptotic activity

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16
Q

Burkitt’s Lymphoma/Leukemia:
- CD markers
- Ki67
- Additional testing that is positive

A

CD45+
B cell markers (CD19, CD22 and CD79a) are all positive
Monoclonal light chain expression of kappa or lambda is positive
CD10+, CD38+, CD43+, CD71+ and bcl-6+
The blast markers CD34 and TdT are negative
Usually Bcl-2−, CD43+

Additional testing reveals expression of PAX-5 (B cell marker) and the Ki-67 (proliferation marker) is expected to be strongly positive. Many cases are EBER (EBV) positive.

17
Q

T lymphoid markers

A

CD3+, CD5+, CD7+, CD1a+, CD2+, CD4+, CD8+
TCR+, CD10+
TdT+

18
Q

B lymphoid markers

A

CD19+, CD10+, CD20+, CD22+, CD24+, cCD79a+
Tdt+ (Moderate to Bright), cyIgM+

19
Q

AML CD markers

A

cMPO+, CD13+, CD33+, CD65+, CD14+, CD15+, CD64+, CD117+

20
Q

CD33+ Favorable Risk AML induction regimen and dosing

A

Standard-dose cytarabine 200 mg/m2 CI x 7 days + Daunorubicin 60 mg/m2 x 3 days &
1 single dose of Gemtuzumab Ozogamicin 3 mg/m2 (up to one 4.5 mg vial) on day 1, 2, 3 or 4

21
Q

Treatment of FLT3 mutated AML:
a. First Line
b. Relapsed/Refractory

A

a. First Line:
Standard dose cytarabine 200 mg/m2 CI x 7 days + Daunorubicin 60 mg/m2 x 3 days (or Idarubicin) & oral midostaurin 50 mg every 12 hours, days 8-21.

b. Relapsed/refractory AML with FLT3 ITD mutation:
–Gilteritinib
–Hypomethylating agent + Sorafenib
–Quizartinib

22
Q

Favorable Risk AML genetic abnormalities

A

t(8;21)(q22.q22.1)
Inv(16) or t(16;16)
Mutated NPM1 without FLT3-ITD
Biallelic mutated CEBPA [bZIP in-frame mutated CEBPA]

23
Q

Intermediate Risk AML genetic abnormalities

A

Mutated NPM1 and FLT3-ITD
Wild-type NPM1 with FLT3-ITD [without adverse-risk genetic lesions]
t(9;11)(p21.3;q23.3)
Cytogenetic abnormalities not classified as favorable or adverse

24
Q

Treatment of refractory FLT3 mutated AML and associated AE

A

Gilteritinib
Adverse Side Effects:
1. Posterior reversible encephalopathy syndrome (PRES)
2. Prolonged QT Interval:
- Interrupt and reduce Gilteritinib in patients who have a QTcF >500 msec
- Correct hypokalemia or hypomagnesemia prior to and during administration.
3. Pancreatitis

25
Q

Maintinance therapy for FLT3 AML and associated indications

A

For patients with AML with FLT3 mutation, the three different consolidation treatment regimens include
–Cytarabine + Midostaurin (FLT3-ITD or TKD)
–Cytarabine + Quizartinib (FLT3-ITD only)
–Allogeneic HCT

If an Allogeneic transplant is performed and a patient with FLT3 mutated AML is in remission, then a FLT3 inhibitor as maintenance therapy should be recommended.

Options include:
–Sorafenib (FLT3-ITD only)
–Midostaurin (FLT3-ITD or TKD; Category 2B)
–Gilteritinib (FLT3-ITD or TKD; Catgeory 2B)
–Quizartinib (FLT3-ITD only)

26
Q

First Line treatment of plasma cell leukemia for fit patients

A
27
Q

Frontline Treatment of mantle cell lymphoma
a. Aggressive treatment regimens (2)
b. Less-aggressive treatment regimens (4)

A

a. Aggressive Treatment Regimens/TP53 mutated:
–Zanubrutinib/Obinutuzumab/Venetoclax
–TRIANGLE regimen with Covalent Bruton’s tyrosine kinase inhibitor + Rituximab

b. Non-aggressive Treatment Regimens/ Non-TP53 mutated
–Rituximab, Dexamethasone, Cytarabine + Platinum (Carboplatin, Oxaliplatin or Cisplatin) x 4 cycles followed
by R-CHOP for non-PET CR
–Alternating R-CHOP + Covalent BTK inhibitor/R-DHA + Platinum
–NORDIC regimen (Dose-intensified induction immunochemotherapy with Rituximab + Cyclophosphamide,
Vincristine, Doxorubicin, Prednisone alternating with Rituximab + High-dose cytarabine)
–Rituximab + Bendamustine followed by Rituximab/High-dose Cytarabine

28
Q

AML favorable risk

A

– t(8;21)
– Inv(16) or t(16;16)
– Mutated NPM1 without FLT3-ITD
– bZIP in-frame mutated CEBPA

29
Q

AML intermediate risk

A

– Mutated NPM1 with FLT3-ITD
– Wild-type NPM1 with FLT3-ITD
– t(9;11)

30
Q

AML high risk

A

t(6;9)(p23;q34.1)/DEK::NUP214
t(v;11q23.3)/KMT2A rearranged (excluding KMT2A-PTD)
t(9;22)(q34.1;q11.2)/BCR::ABL1
(8;16)(p11;p13)/KAT6A::CREBBP
inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2)/GATA2, MECOM(EVI1)
t(3q26.2;v)/MECOM(EVI1)-rearranged
−5 or del(5q); −7; −17/abn(17p)
Complex karyotype (change in definition)e; Monosomal Karyotype
Mutated ASXL1, BCOR, EZH2, RUNX1, SF3B1, SRSF2, STAG2, U2AF1, or ZRSR2f
Mutated TP53 (Variant Allele Frequency ≥10%)

31
Q

AML with FLT3 mutation:
First Line + associated AE
Second Line + associated AE

A

First Line- AraC + Midosaurin (AE: nausea, GI)
Second Line
– Gliteritinib (AE: PRES, QTC prolongation, pancreatitis)
– Quizartinib (AE: WBC, QTC prolongation)

32
Q

AML with IDH1/2 mutations

A

IDH1 mutation:
AZA 75 mg/m2 qD SC/IV d1-7 (alternatively d1-5 + d8-9) + Ivosidenib 500 mg PO QD d1-28
– If unable to tolerate chemotherapy, can be treated with Ivosidenib mono therapy

IDH2: Enasidenib: 100 mg PO QD d1-28, q4 weeks, until disease progression

33
Q

2nd Line/Treatment of relapsed/refractory mantle cell lymphoma

A

Covalent BTKis (acalabrutinib and zanubrutinib) and lenalidomide + rituximab

34
Q

Mantle cell lymphoma typical immunophenotype
- positive stain if unclear diagnosis?
- FISH if unclear diagnosis

A

Immunophenotype:
CD5+, CD20+, CD43+, cyclin D1+, CD10-/+

Stain: Cyclin D1

FISH: t (11, 14 )

Oncology Boards (13 decks)

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